Charcot-Marie-Tooth disease at first always sounds
like a problem with teeth. It is not
however, it was named for three neurologists Charcot, Marie and Dr. Howard Tooth who first
clinically described in in 1886.

It is apparently an inherited neurological disease,
and is referred to as a hereditary neuropathy (nerve
damage). It affects both motor and sensory
nerves. It is referred to as Charcot-Marie
Tooth syndrome, CMT, hereditary motor and sensory neuropathy
HNSN, and Peroneal Muscular Atrophy, PMA.

There is also a
condition named Charcot Foot. You can read
about it in this article Charcot
Foot. It is connected to neuropathy and is
talked about on this page Neuropathy.

The periphery nerves - the ones affected - are
located outside the main central nervous system.
Periphery nerves control muscles, as well as relaying
data from our arms and legs to our brain, which allow
us to sense touch. Charcot-Marie-Tooth disease (CMT)
involves muscle weakness and loss of sensation,
predominantly in the arms and legs, especially in the
calves.

This disease affects the periphery nerves, which
control muscles and send messages from the legs and
arms to the brain. These messages give us our sense of
touch. The disorder generally makes itself known
in childhood or adolescence, though this can happen at
any age.

Symptoms of Charcot Marie Tooth Disease:

The earliest indicators of CMT are usually
clumsiness and weakness in the ankles, feet and legs.
The feet may be either extremely arched or extremely
flat, and the toes may be curled upwards, into
"hammertoes". Walking can be awkward
due to the weak ankle muscles. It causes the
individual to step higher than normal; the toes may
drop forward.

As with any neuropathy, arms and feet become numb.
Injuries can occur due to lack of sensation.

Symptoms are:

Weakness in the muscles of the lower feet, legs
and ankles

Ankle becomes unstable because the feet are very
highly arched, or less commonly, very flat.

Hammertoes (curled toes)

Lifting up of the foot is difficult because of
weak ankle muscles (footdrop)

A step (stride) which is higher than normal (an
awkward step/gait)

Numbness in the arms and feet

Changing leg shape - the section of the leg
below the knee becomes very thin, while the thighs
retain their normal muscle volume and shape (stork
legs)

Cramping can occur in the lower legs and forearms,
and some of the body's reflexes can deteriorate. The
hands suffer weakness and atrophy in the muscles and
fine motor ability decreases.

Balance is affected due to the motor nerves being
damaged. The spine may curve, scoliosis. There
may be some hearing or sight loss depending on the
nerves affected.

Symptoms may vary. Some people will have mild
symptoms that do not interfere with their ability to
live normal lives. Very often though, numbness and
weakness will have far-reaching effects.

CMT does not affect life expectancy. As
with any nerve damage, there can be progressive muscle
weakness and other neuropathy symptoms.

Progressive signs and symptoms may include:

The patient's hands and arms become more
affected.

Problems with manual dexterity, such as doing up
buttons, or opening jars and bottle tops.

Muscle and joint pain resulting from problems
with walking and posture which place a strain on
the body.

Neuropathic pain cause by damaged nerves (less
common).

General walking and mobility problems,
especially with older patients.

Causes:

There are two parts of the nerves"

The axon - the inside of the nerve. Like
the metal part of an electric cable. The
electrical information between the brain and the
limbs passes through the axon, in the same way
electricity passes through the metal part of a
cable (wire).

The myelin sheath - the insulation around
the axon, in the same way a wire/cable may have
some kind of plastic insulation around it. The
myelin sheath wraps around the axon, protecting
it, as well as helping restore the quality of the
electrical signal.

Either the axon or myelin sheath, or both, may be
affected when a patient has CMT - it depends on the
type. Mutated (faulty) genes cause the disintegration
of the myelin sheath. If the myelin sheath wastes away
it becomes thinner and thinner, eventually the axon
becomes damaged and the patient's muscles no longer
receive clear messages from the brain, resulting in
muscle weakness and loss of sensation (numbness).

In some types of CMT the axons are directly targeted;
also as a result of faulty genes. For some reason the
electrical signals are not transmitted at the required
strength to activate muscles and senses, resulting in
weaker muscles and poorer tactile sensitivity
(numbness).

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